Dubas Vitalii, Buch Maya H, Seferovic Petar, Matucci-Cerinic Marco, Bruni Cosimo
Department of Noncoronary Heart Diseases, Rheumatology and Therapy, State Institution, National Scientific Center, The M.D. Strazhesko Institute of Cardiology, Clinical and Regenerative Medicine of the National academy of Medical Sciences of Ukraine, Kyiv, Ukraine.
Centre for Musculoskeletal Research, Division of Musculoskeletal & Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK.
J Scleroderma Relat Disord. 2025 Mar 19:23971983251321691. doi: 10.1177/23971983251321691.
The aim of this study was to evaluate the level of agreement among international experts on the World Scleroderma Foundation/Heart Failure Association consensus-based definition of systemic sclerosis-associated primary heart involvement (SSc-pHI) and on the guidance for its screening, diagnosis, and follow-up assessment, including feasibility and applicability assessments.
An online survey was conducted to assess the level of agreement and feasibility/applicability using a 10-point scale (0 = , 10 = ). The weblink to the survey was shared with 1199 participants worldwide. A high level of agreement was defined by an average score over 7/10.
In total, 161 external experts completed the survey. Most of them were rheumatologists (80.7%), working in Europe (81.4%), and had > 10 years of clinical experience in managing SSc patients (59%). Overall agreement among external experts was high (mean 8.27, SD 1.86). The highest scores regarded items emphasizing the involvement of a multidisciplinary team, personalization of patient management, and initial evaluation techniques. A lower level of agreement was obtained in questions related to cardiac magnetic resonance imaging and endomyocardial biopsy. No factors associated with low level of agreement and feasibility/applicability were identified.
The consensus-based definition and guidance on screening, diagnosis, and follow-up assessment of SSc-pHI provides a basis for application by the wider community. A lower level of agreement on the use of advanced or more invasive diagnostic techniques likely reflects regional differences in access and the need for more evidence on its use. This emphasizes the importance of involving a multidisciplinary team.
本研究旨在评估国际专家对于世界硬皮病基金会/心力衰竭协会基于共识的系统性硬化症相关原发性心脏受累（SSc-pHI）定义以及其筛查、诊断和随访评估指南（包括可行性和适用性评估）的一致程度。
开展了一项在线调查，使用10分制量表（0 = ，10 = ）评估一致程度以及可行性/适用性。调查的网络链接分享给了全球1199名参与者。平均得分超过7/10被定义为高度一致。
共有161名外部专家完成了调查。他们大多数是风湿病学家（80.7%），在欧洲工作（81.4%），并且在管理硬皮病患者方面有超过10年的临床经验（59%）。外部专家的总体一致程度较高（平均8.27，标准差1.86）。得分最高的项目是强调多学科团队参与、患者管理个性化以及初始评估技术的项目。在与心脏磁共振成像和心内膜心肌活检相关的问题上，一致程度较低。未发现与低一致程度以及可行性/适用性相关的因素。
基于共识的SSc-pHI筛查、诊断和随访评估定义及指南为更广泛的群体提供了应用基础。对于先进或更具侵入性诊断技术使用的较低一致程度可能反映了获取途径的地区差异以及对其使用需要更多证据。这强调了多学科团队参与的重要性。
